title: Epidermolysis bullosa acquisita: a retrospective clinical analysis of 30 cases.
abstract: Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune blistering disorder caused by autoantibody production against type VII collagen. The aim of this study was to examine the clinical types, treatments, and outcomes of 30 patients with EBA. In our cohort, the median age of onset was 44.0 years, with a similar incidence for both genders (46.7% male, 53.3% female). The majority of patients had classic type (36.7%) and bullous pemphigoid (BP)-like type (46.7%) EBA. The remaining patients had mucous membrane pemphigoid-like (6.7%), Brunsting-Perry pemphigoid-like (6.7%), and linear IgA bullous dermatosis-like type (3.3%) EBA. All patients were treated initially with a combination of methylprednisolone, dapsone and colchicine. No significant differences in time to remission were identified between patients with classic vs. BP-like EBA. In a second subset analysis of 19 patients, a group treated with high-dose (> 8 mg) methylprednisolone achieved remission earlier (median time to remission: 3 months) than a group treated with low-dose (≤ 8 mg) methylprednisolone (median time to remission: 12 months), irrespective of clinical type (p = 0.003).
authors.affiliation: Department of Dermatology, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jong Hoon
authors.initials: JH
authors.lastname: Kim
authors.forename: Yeon Hee
authors.initials: YH
authors.lastname: Kim
authors.forename: Soo-Chan
authors.initials: SC
authors.lastname: Kim
mesh_terms: D000328:Adult; D000368:Aged; D003078:Colchicine / Q000008:administration & dosage; D003622:Dapsone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D016107:Epidermolysis Bullosa Acquisita / Q000145:classification / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D053208:Kaplan-Meier Estimate; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D012074:Remission Induction; D056910:Republic of Korea; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D003622:Dapsone; D003078:Colchicine; D008775:Methylprednisolone